Conclusion 4
The clinician needs to have a high index of suspicion for pancreatitis in the child who presents with the non-specific but common symptoms of nausea, vomiting and abdominal pain. A thorough history that emphasizes recent infections, medications, trauma and any underlying medical condition may make the diagnosis clearer. The traditional use of enzyme testing alone serum amylase and lipase for the diagnosis of both acute and chronic pancreatitis may not be adequate, since the clinical specificity...
Conclusion 3
Most of the knowledge and tools needed to prevent diarrhea-associated mortality in developing countries and especially persistent diarrhea are available. These require concerted and sustained implementation in public health programs.62 Given the emerging evidence of the long-term impact of childhood diarrhea on developmental outcomes,63 it is imperative that due emphasis is placed on prompt recognition and appropriate management of persistent diarrhea. 1. Black RE, Morris SS, Bryce J. Where and...
Diagnosis
Diagnosis of IED is sometimes difficult for several reasons. The early onset of severe and permanent diarrhea and the lack of features diagnostic of microvillus inclusion disease are important diagnostic elements. However, the characteristic tufts of extruding epithelium may not be obvious, especially early on. At the onset of the clinical course, IED is most often suspected after elimination of MVID, and the final diagnosis is made rather late, by performing repeated intestinal biopsies which...
Principles of management of persistent diarrhea
In general the management of persistent diarrhea in malnourished children represents a blend of the principles of management of diarrhea and malnutrition. Associated malnutrition may be quite severe in affected children, necessitating rapid nutritional rehabilitation, often in hospital. Given This is the preferred mode of rehydration and replacement of on-going losses. While in general the standard WHO oral rehydration solution ORS is adequate, recent evidence indicates that hypo-osmolar...
Stress gastropathy
Although the overall prevalence is unknown, most critically ill infants and children are prone to develop stress-related gastropathy. In infants, stress gastropathy is usually related to traumatic delivery, respiratory or cardiac failure, sepsis, hypoglycemia or dehydration, while in older children it is related to severe life-threatening illness e.g. respiratory or cardiac failure , intracranial lesion, trauma, burns, coagulopathy, or vasculitis.23 Endoscopic features include isolated...
Diagnosis 6
The list of conditions that should be considered in the differential diagnosis for Crohn's disease is extensive, and is related to the various clinical presentations of Crohn's disease Table 23.6 . A high index of clinical suspicion is crucial if the clinician is to make a diagnosis in the early phases of the illness. Delay in growth is often a clue that the presenting symptoms are not caused by an acute illness. Clinical suspicion for Crohn's disease It is important to rule out Crohn's disease...
Contents
1 Microvillus inclusion disease and 1 epithelial dysplasia 2 Congenital problems of the 13 gastrointestinal tract Nigel Hall and Agostino Pierro II Diseases of the Esophagus and Stomach 3 Infectious esophagitis 29 Salvatore Cucchiara and Osvaldo Borrelli 4 Gastroesophageal reflux disease 39 Yvan Vandenplas, Silvia Salvatore and Bruno Hauser 5 Achalasia 61 Carl-Christian A Jackson and Donald C Liu 6 Helicobacter pylori gastritis and 73 peptic ulcer disease Costantino De Giacomo 7 Other...
Chronic granulomatous disease
Chronic granulomatous disease is a rare group of inherited disorders characterized by impaired phagocyte oxidative metabolism caused by missing components or subunits of the NADPH oxidative complex, resulting in defective intracel-lular killing of catalase-positive micro-organisms. The most common form of chronic granulomatous disease is inherited as an X-linked recessive trait, although autosomal-type mutations have been described. The disease usually appears during the first two years of life...
The basic mechanisms of immune response to dietary antigen
The intestine is an organ that shows the traces of evolutionary longevity, and indeed Cambrian period fossils from over 600 million years ago show a recognizable gastrointestinal tract.118 There is much current interest on the links between innate and adaptive immune responses, in particular pattern receptor molecules such as toll-like receptors and nod proteins that induce an immune response within innate cells, such as dendritic cells, that polarize subsequent T-cell responses.119,120...
Treatment 17
Only a few studies, most of them with small patient numbers, have been performed to evaluate treatment for children with defecation disorders. Treatment of constipation is mainly based on empirical experience, rather than on placebo-controlled, randomized studies. The main reason for a consultation is interference with social activities of the child and its family because of the defecation problems soiling and encopresis . They should be educated and the problem should be demystified.29 It...
Constipation in mentally handicapped children
The incidence of constipation was around 61 in a large cohort of mentally handicapped children in Dutch and Belgian institutions. Constipation was defined as bowel movements less than 3 times a week. Eighty-eight per cent of the constipated, mentally handicapped, children used laxatives, in comparison to 40 of constipated controls whose constipation was easily controlled. A significant correlation was found between non-ambulancy, cerebral palsy, use of anticonvulsive medication or...
Pathogenesis
Role of acid and pepsinogen secretion The bulk of our knowledge on acid secretion is derived from studies in adults. Gastric acid secretion begins in the newborn from the first day of life it is not sensitive to gastrin, but rather to pentagastrin stimulation, which is the best way to evaluate it. Serum gastrin is usually elevated in the newborn period.35,36 Maximal acid output after pentagastrin stimulation increases from 0.031 mEq kg per h at 1 month to 0.122 mEq kg per h at 3 months, and up...
Trichuriasis
Trichuris trichiuria, meaning 'hairy tail', is actually a misnomer, since it is the proximal end that is hairlike. The popular name is whipworm, with the whip as the long thin pharynx stichostome and the whip handle as the posterior end with reproductive organs and intestine Figure 11.3 . A mature female worm produces up to 20 000 eggs day, which are 50 im long and not infectious until the larval stage develops in the soil over 2-4 weeks. Warm damp soil is ideal for embryonation, Figure 11.3...
Meconium ileus
Meconium ileus is a common cause of neonatal intestinal obstruction and the most common cause of antenatal intestinal perforation.19 It should be included in the differential diagnosis of infants presenting with GI tract obstruction. In approximately 80 of cases it is associated with cystic fibrosis.20-22 The underlying defect in cystic fibro-sis, an abnormality in a transmembrane chloride channel, results in the production of abnormally viscid and sticky meconium. This meconium sticks to the...
Shigella
Kiyoshi Shiga first isolated Shigella dysenteriae type 1 during a severe dysentery epidemic in Japan in 1896, when more than 90000 cases were described with a mortality rate approaching 30 .18 Shigellae are Gram-negative, non-lactose fermenting, non-motile bacilli, with S. sonnei the main type found in industrialized countries, and S. flexneri and S. dysenteriae predominating in underdeveloped countries. Humans are the only natural hosts and transmission occurs by fecal-oral contact. The low...
Portal hypertensive gastropathy
Involvement of the gastric mucosa is common in children with intrahepatic or extrahepatic causes of portal hypertension.33 Endoscopic findings vary from mild involvement, including a snake-skin mosaic pattern of the mucosa, a fine pink speckling and superficial erythema scarlatina-type rash , to a severe gastropathy, defined by cherry red spots with a diffuse confluence of reddened areas and a hemorrhagic appearance. These patterns seem to be specific for portal hypertensive gastropathy and...
Classification
A number of classification systems have been proposed over the years. The abbreviated list in Table 2.1 describes the most commonly encountered anatomical variants Figure 2.1 . The Table 2.1 Classification of esophageal atresia EA tracheoesophageal fistula TEF anomalies and frequency6 Figure 2.1 Common anatomical variants of esophageal atresia EA tracheoesophageal fistula TEF anomalies. a EA with distal TEF b isolated EA with no TEF c H-type TEF d proximal and distal TEF e EA with proximal TEF....
Pathophysiology and mode of transmission
A defect in the membrane trafficking of immature and or differentiating enterocytes has been discussed as an etiopathogenic mechanism in MVID.20,21 This membrane defect results, as a Figure 1.2 Microvillus inclusion disease CD-10 immunostaining. a Normal mucosa, normal CD-10, brush-border immunostaining b abnormal accumulation of positive material in the apical cytoplasm of epithelial cells in microvillus inclusion disease. direct functional consequence, in complete intestinal failure. It has...
Other parasites Hymenolepiasis
Hymenolepis nana is the dwarf tapeworm and the only human tapeworm that does not require an intermediate host. Nevertheless, rodent strains for H. nana are infectious for humans including pet rodents such as rats, mice and hamsters. It occurs worldwide, with high childhood prevalences gt 10 reported from Argentina, Peru, Brazil, Egypt, Pakistan and Zimbabwe.41 Transmission is mostly fecal-oral, either from person to person or in food and water, with high rates in children from orphanages.139...
Nonpharmacological and nonsurgical therapies for gastroesophageal reflux
Non-pharmacological and non-surgical therapies for reflux do not have any proven efficacy on reflux, although some may decrease the incidence of regurgitation. Lifestyle changes in adults are rarely beneficial.96 No significant difference was shown between the flat and head-elevated prone position. Despite gravity, the upright seated position leads to significantly more and larger reflux episodes than the simple prone and 30 elevated prone position, when the infant is awake or asleep.97 This is...
Histological features
Villus atrophy of variable severity is present. In the typical form, abnormalities are localized mainly in the epithelium and include a disorganization of surface enterocytes with focal crowding, resembling tufts Figure 1.5 . These characteristic 'tufts' of extruding epithelium first described by Reifen et al36 are seen towards the villus tip, and may affect up to 70 of villi. The tufting process is not limited to the small intestine but also involves the colonic mucosa.11 This picture can also...
Gastrointestinal duplications
Duplication cysts of the GI tract are rare congenital abnormalities. They can occur at any point in the GI tract from mouth to anus, although they are most commonly found around the ileocecal region. Duplication cysts are defined according to strict criteria, as devised by Ladd and Gross they are closely attached to some part of the GI tract, have a smooth muscle coat and have an epithelial lining that resembles some part of the alimentary canal.43 Duplications may be spherical or tubular in...
Domperidone
The studies supporting efficacy of domperidone in improving GER in infants are limited.104 The ability of oral domperidone to increase the pressure of the LES or to promote healing of reflux esophagitis has not been demonstrated in placebo-controlled trials. Most studies have been performed in older children, or investigate the effects of domperidone co-administered with other anti-reflux agents.104 Comparing domperidone to metoclopramide, elicited adverse effects on the central nervous system...
Amebiasis
Recent molecular and immunological techniques have demonstrated two distinct species of Entamoeba that are morphologically identical. E. histolytica is pathogenic, causing symptomatic disease in 10 of infections whereas E. dispar causes only asymptomatic colonization. In addition to the E. histolytica strain, other risk factors for invasive disease are interaction with bacterial flora, host genetic susceptibility, malnutrition, male sex, young age and immunodeficiency. Entamoeba coli and E....
Diagnosis 2
Diagnosis of ulcer is based on the endoscopic examination of the stomach and the duodenal Figure 6.3 Endoscopic appearance of a Helicobacter pylori-associated peptic ulcer of the posterior wall of the duodenal bulb in an 11-year-old girl. Figure 6.3 Endoscopic appearance of a Helicobacter pylori-associated peptic ulcer of the posterior wall of the duodenal bulb in an 11-year-old girl. Figure 6.4 Endoscopic appearance of Helicobacter pylori-associated multiple peptic ulcers of the gastric body...
Campylobacter
These organisms are small, microaerophilic, spiral-shaped Gram-negative organisms that enjoy a widespread reservoir in the intestines of both wild and domestic animals.21 It is the most frequently identified bacterial cause of diarrhea in the USA. Common vehicles are poultry, un-pasteurized milk and contaminated water.22-24 After an incubation of 3-6 days, enteritis begins abruptly with cramps and watery diarrhea, which may progress to blood-containing stools. The abdominal pain may mimic...
Muscular dystrophy
Gastrointestinal involvement may occur in a number of forms of muscular dystrophy including myotome muscular dystrophy and the dystro-phinopathies Duchenne and Becker muscular dystrophy. Involvement of the smooth muscle of the gastrointestinal tract and bladder is well described in myotonic muscular dystrophy, and seemingly motor abnormalities can be found throughout the whole gastrointestinal tract.23 Duchenne and the milder variety Becker muscular dystrophy are due to abnormalities of...
Classification 1
There are four basic types of duodenal obstruction Figure 2.4 . In type 1 there is a stenosis of the duodenum resulting from a diaphragm or web partially or totally occluding the lumen. Owing to the incomplete nature of the obstruction, cases Duodenal atresia, duodenal stenosis and annular pancreas are the most common congenital condi- Figure 2.3 Upper gastrointestinal contrast study of a case of malrotation. The contrast is seen within the duodenum D and flowing into the upper jejunum J , both...
Etiology 4
The disease processes, which result in CIP, affect the control mechanisms of intestinal motility. The disorders and disease may primarily be of the intrinsic enteric nerves with or without involvement of the extrinsic autonomic nerves, the smooth muscle cells themselves or of the tumoral and endocrine environment. Good examples of the effect of disturbance of the endocrine environment are the ileus associated with vasoactive intestinal polypeptide-secreting tumors such as ganglioneuromas, and...
Menetriers disease
Menetrier's disease, a rare disorder of unknown etiology, is characterized by enlarged gastric folds due to mucosal thickening in the gastric body. To date, fewer than 100 pediatric cases have been described. Significant differences between adult and pediatric disease in terms of onset, presentation, course and prognosis have been observed.25,26 In children, the disease often begins abruptly and is usually self-limited, with resolution of clinical features within weeks or months. In contrast,...
Signs and symptoms
Because the incidence of achalasia in children is so low, the majority of information regarding the diagnosis comes from adults. The earliest, and most common, symptom of achalasia is dysphagia. This dysphagia initially is for solids, but frequently progresses to dysphagia for liquids by the time treatment is sought.65-67 The next most common symptom in adults is regurgitation, which is non-acidic and non-bilious, owing to the contracted LES, and most often occurs right after eating or during...
Classification 2
Morphological classification of these lesions allows different surgeons and centers to compare outcomes and is also of therapeutic and prognostic value. The most commonly accepted system is that proposed by Louw13 and modified by Grosfeld et al.14 Whether the lesion is classified as ileal or jejunal is determined by the most proximal affected segment Figure 2.5 . Stenosis is a localized narrowing of the lumen without any break in the continuity or mesenteric defect. The intestinal wall may be...
Fungal infections
Candida species are the most common agents of infectious esophagitis. Candida albicans is the most common pathogen, but C. tropicalis, C. parapsilosis and C. glabrata have occasionally been reported. These organisms are usually Table 3.1 Causes of esophageal disease in HIV infection and AIDS present in the normal oral flora, where their growth is controlled by commensal organisms. Conditions predisposing to esophageal candidiasis in immunocompetent subjects are inhaled or ingested...
Prognosis 1
In a cohort of more than 400 constipated children, follow-up was obtained by annual telephone contact in more than 95 of children. Mean follow-up was 5 years range 1-8 . The cumulative percentage of children who were successfully treated during follow-up was 60 in 1 year, increasing to 80 in 8 years Figure 16.1 . Interestingly, successful treatment was more Figure 16.1 Outcome with and without laxatives. Figure 16.1 Outcome with and without laxatives. frequent in children without encopresis,...
Treatment 6
Treatment in the first instance is aimed at decompression of the distal GI tract by regular rectal washouts. Subsequently a number of surgical options exist, all of which aim to remove the agan-glionic segment and restore intestinal continuity by means of an anastomosis of ganglionated bowel to the rectal stump so-called 'pull-through' . This may be performed either as a primary procedure or as a delayed procedure after initial colostomy formation Figure 2.6 Plain abdominal X-ray of a child...
Cholera
Of all enteric pathogens, Vibrio cholerae is responsible for the most rapidly fatal diarrheal disease in humans.1 Although cholera is rare in developed countries, it remains a major cause of diarrheal morbidity and mortality in many parts of the developing world.2 However, with the occurrence of both natural e.g. earthquakes and human-generated calamities such as ethnic wars , the spreading of cholera infection in refugee camps, where sanitary conditions resemble those in cholera endemic areas,...
Laboratory and instrumental investigations
For patients in whom a diagnosis is uncertain, a plain chest radiograph may be the first available study. The classically described finding on upright chest X-ray is an absent gastric air-bubble. This finding, present in nearly all normal individuals, is absent in approximately half of patients with acha-lasia.73,74 Additional findings include a widened mediastinum from esophageal dilatation, a posterior mediastinal air-fluid level from retained food secretions and lung parenchymal...
Crohns disease
Although Crohn's disease CD most commonly affects the terminal ileum and or the colon, involvement of the upper gastrointestinal tract is frequently found in both adults and children. Symptoms such as epigastric pain, early satiety, nausea, vomiting, weight loss and, less frequently, hematemesis and melena are commonly reported in these patients and have previously been related to reflex inhibition of foregut motility secondary to inflammation and partial obstruction of the distal small bowel....
Histological examination
Histological analysis of small bowel biopsies shows a variable degree of villus atrophy without any inflammatory infiltrate. Highly characteristic for this disorder is the accumulation of periodic acid Schiff PAS -positive secretory granules within the apical cytoplasm of enterocytes.18-20 On the ultrastructural level, rare or absent microvilli on intestinal epithelial cells along with inclusions of microvilli in the cytoplasm of enterocytes are seen, which define this entity.21 Diagnosis may...
Collagenous gastritis
Collagenous gastritis is an extremely rare disorder of unknown etiology, To date, fewer than ten cases have been reported in the literature. The condition is characterized by deposition of a subepithelial collagen band greater than 10 im in thickness. The disorder was originally described by Colletti and Trainer in a 15-year-old girl with refractory H. pylori-negative chronic gastritis.55 It has been reported either as an isolated entity or with synchronous collagenous colitis, collagenous...
Complications 5
Complications of ulcerative colitis include massive hemorrhage, toxic megacolon, perforation of the bowel, strictures and colon cancer. Massive hemorrhage can occur with severe ulcerative colitis and is managed with blood transfusions and treatment of the underlying ulcerative colitis urgent colectomy may be required. One consensus group suggested that an individual with ulcerative colitis who requires more than 6-8 units of blood in the first 48h and is still actively bleeding should undergo a...
Ascariasis
Ascaris lumbricoides is a large, 15-35 cm long white roundworm that is specific to humans. Ascaris is one of 63 species of nematode infecting humans, and the adult roundworm has a biologically inert surface, so the main antigenic stimuli to the host are excretory and secretory antigens from the orifices. A. suum is the related pig species which may migrate through human tissues in the larval stage,36 but cannot complete its life cycle in man. The female Ascaris worm produces about 200 000 eggs...
Pathophysiology
The pathophysiology of GER is complex and diverse, as it is influenced by factors that are genetic, environmental e.g. diet and smoking , anatomic, hormonal and neurogenic Figure 4.1 and Table 4.1 . We have recently reviewed the Gastric distension gastric mechanoreceptors near cardia Vagally mediated abnormal neural control of LES by CNS Defective LES motility Increased TLESRs - lt Low basal LES tone - lt - Gastric acid Hiatal hernia, obtuse angle of His Delayed acid clearance Delayed volume...
Risk factors for persistent diarrhea
It is important to recognize the major risk factors for development of persistent diarrhea, as appropriate case management of acute diarrhea is key to the prevention of prolonged episodes. The association of specific bacterial and viral infections with persistent diarrhea has been the subject of considerable debate.30,31 Evidence from Risk factors for persistent diarrhea 195 Figure 12.2 Mechanisms and effects of enteropathy of malnutrition and prolonged diarrhea. Figure 12.2 Mechanisms and...
Strongyloidiasis
Although not a major cause of morbidity worldwide, the nematode Strongyloides stercoralis is unique in its ability to persist indefinitely within the host through autoinfection, and cause disseminated disease with the prolonged use of cortico-steroids or other causes of immunosuppression. Adult females are about 2.5 mm in length and are attached to the lamina propria of the duodenum or proximal jejunum Figure 11.4 . Their eggs, which hatch into rhabditiform larvae 250 im long and pass via feces...
Clinical signs and symptoms 1
The initial clinical presentation of Crohn's disease may be subtle, variable, non-specific and easily overlooked. Common symptoms include abdominal pain, diarrhea and or weight loss.103 A plateau in linear growth, delayed pubertal development, perianal lesions, fever, pallor, hematochezia and digital clubbing may also be present.103 Crohn's disease may affect any area of the gastrointestinal tract from the lips to the perianal area. In a study on Scottish children and adolescents with Crohn's...
Diagnosis with differential 1
For the diagnosis of Hirschsprung's disease, the subject's history is very important. The crucial elements to obtain are the age of the appearance of symptoms whether the passage of meconium has been normal or delayed and whether the child presented with episodes of functional intestinal obstruction. In addition, a functional idiopathic megacolon must be ruled out. A clinical comparison between functional and congenital megacolon is shown in Table 17.1. When the history early onset of...
Pathophysiology of viral diarrhea
In the classic and simple view, the pathogenesis of diarrhea may be divided into osmotic and secretory Figure 9.1 . Viral diarrhea was originally believed to be caused by cell invasion and epithelial destruction by enteropathogenic agents, therefore being the result of endoluminal fluid accumulation osmotically driven by non-absorbed nutrients. It is now known that several mechanisms are responsible for diarrhea, depending on the specific agents and the host features. In addition, selected...
Intestinal malrotation
The incidence of intestinal malrotation is difficult to establish as not all affected patients develop symptoms, but autopsy studies estimate the incidence at approximately 1 in 500. The traditional embryological basis for disorders of intestinal rotation is that of abnormal position ing of the intestinal loops in relation to one another as they return to the abdominal cavity from the yolk sac. During normal development the midgut rotates through 270 so that the duodenum lies posterior to the...
Yersinia
Yersinia enterocolitica and Y pseudotuberculosis are two important human enteropathogens distributed widely in the environment, with swine serving as the major reservoir. The incubation period is 3-7 days, with food-borne transmission the suspected route for most infections. Yersinia's preference for cool temperatures makes this pathogen more common in Northern Europe, Scandinavia, Canada, the USA and Japan, where it is responsible for up to 8 of sporadic diarrhea episodes. Yersinia...